{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T22:32:54Z","timestamp":1775514774680,"version":"3.50.1"},"reference-count":0,"publisher":"Stichting Nase","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rhin"],"published-print":{"date-parts":[[2011,8,1]]},"abstract":"<jats:p>The aim of this study was to address whether NP might be a predictive factor for severity of CF. The authors collected data from the literature on NP as a unique or associated sign in CF and reviewed the clinical and molecular aspects of CF associated with NP. CF genotypes and clinical severity in NP(+) vs. NP(-) patients were reviewed, taking into account pulmonary function, frequency of P. aeruginosa lung infection, frequency of allergy, nutritional status, and exocrine pancreatic function. The CFTR gene was also analyzed in a patient with isolated severe NP as the unique feature of CF. This review of the literature showed a `milder` phenotype in `NP+` vs. `NP-` CF patients, contrasting with a marked association between NP and `severe` CF mutations. In addition, a complex genotype was identified, associating four heterozygous variants, namely p.Q493X (a severe mutation) on the paternal allele, and p.V562I, p.A1006E, and (TG)11(T)5 (IVS8-5T) on the maternal allele, in a case of CF presenting as isolated NP. The authors speculate that genetic\/environmental factors associated with NP might attenuate the functional impact of `severe` CF mutations. The overrepresentation of CF carriers among patients with isolated NP also advocates the need for CFTR molecular screening in such populations for genetic counselling purposes.<\/jats:p>","DOI":"10.4193\/rhino10.225","type":"journal-article","created":{"date-parts":[[2021,5,6]],"date-time":"2021-05-06T10:16:19Z","timestamp":1620296179000},"page":"347-355","source":"Crossref","is-referenced-by-count":26,"title":["Nasal polyposis and cystic fibrosis(CF): review of the literature"],"prefix":"10.4193","volume":"49","author":[{"given":"M.N.","family":"Feuillet-Fieux","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Lenoir","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Sermet","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Elie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Djadi-Prat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Ferrec","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Magen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"Couloigner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Manach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Lacour","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.P.","family":"Bonnefont","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2708","container-title":["Rhinology journal"],"original-title":[],"deposited":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T15:34:23Z","timestamp":1621956863000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.rhinologyjournal.com\/Abstract.php?id=1010"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,8,1]]},"references-count":0,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2011,8,1]]}},"URL":"https:\/\/doi.org\/10.4193\/rhino10.225","relation":{},"ISSN":["0300-0729"],"issn-type":[{"value":"0300-0729","type":"print"}],"subject":[],"published":{"date-parts":[[2011,8,1]]}}}